Results 31 to 40 of about 8,574 (264)
The presence of valine at residue 129 in human prion protein accelerates amyloid formation [PDF]
The polymorphism at residue 129 of the human PRNP gene modulates disease susceptibility and the clinicopathological phenotypes in human transmissible spongiform encephalopathies.
Tahiri-Alaoui, Abdessamad +13 more
core +1 more source
Anti-prion drug mPPIg5 inhibits PrP(C) conversion to PrP(Sc). [PDF]
Prion diseases, also known as transmissible spongiform encephalopathies, are a group of fatal neurodegenerative diseases that include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans.
Jeremy C. Simpson (29225) +31 more
core +2 more sources
How susceptible pigs are to infection with sheep prions is unknown. We show, through transmission experiments in transgenic mice expressing porcine prion protein (PrP), that the susceptibility of this mouse model to bovine spongiform encephalopathy (BSE)
Juan-Carlos Espinosa +8 more
doaj +1 more source
Chronic wasting disease (CWD) is a naturally-occurring neurodegenerative disease of cervids. Raccoons (Procyon lotor) and meadow voles (Microtus pennsylvanicus) have previously been shown to be susceptible to the CWD agent.
S. Jo Moore +4 more
doaj +1 more source
Chronic Wasting Disease Prions in Elk Antler Velvet
Chronic wasting disease (CWD) is a contagious, fatal prion disease of deer and elk that continues to emerge in new locations. To explore the means by which prions are transmitted with high efficiency among cervids, we examined prion infectivity in the ...
Rachel C. Angers +8 more
doaj +1 more source
The anti‐prion RNA aptamer R12 disrupts the Alzheimer's disease‐related complex between prion and amyloid β [PDF]
The neurodegenerative disorder Alzheimer's disease (AD) is associated with the accumulation of misfolded proteins. Some recent studies suggested that amyloid beta (Aβ) forms soluble oligomers, protofibrils, and fibrils; the Aβ oligomers being more toxic than the fibrils.
Mamiko, Iida +5 more
openaire +2 more sources
Infectious, self-propagating protein aggregates (prions) as well as structurally related amyloid fibrils have traditionally been associated with neurodegenerative diseases in mammals.
Weissman, Jonathan S. +1 more
core +1 more source
A Case–Control Study on the Origin of Atypical Scrapie in Sheep, France
A matched case–control study (95 cases and 220 controls) was designed to study risk factors for atypical scrapie in sheep in France. We analyzed contacts with animals from other flocks, lambing and feeding practices, and exposure to toxic substances ...
Alexandre Fediaevsky +3 more
doaj +1 more source
Prion-related peripheral neuropathy in sporadic Creutzfeldt-Jakob disease
ObjectiveTo assess whether the involvement of the peripheral nervous system (PNS) belongs to the phenotypic spectrum of sporadic Creutzfeldt-Jakob disease (sCJD).MethodsWe examined medical records of 117 sCJDVV2 (ataxic type), 65 sCJDMV2K (kuru-plaque type) and 121 sCJDMM(V)1 (myoclonic type) subjects for clinical symptoms, objective signs and ...
Baiardi S. +14 more
openaire +3 more sources
Exploring the mechanisms involved in prion degradation and spreading from cell-to-cell in neuronal cell models [PDF]
Transmissible spongiform encephalopathies (TSE), also known as prion diseases, are fatal neurodegenerative disorders present both in human and animals with different aetiology as they can occur genetically, spontaneously or by infection (Prusiner 1998 ...
Marzo, Ludovica
core +1 more source

